Please find it in your hearts to spare a few minutes of your time to read this. Enclosed is a story that not only needs to be told but must be heard in order to make a difference. Frank and Nadine (DiMezza) Accurso and their Family thank you in advance for caring enough to read what we have written. This has been passed on to you in an effort to expand public knowledge and to give exposure to an otherwise unknown disease. By no means can we sum up everything in one passage; however, we hope to give you some understanding and insight.
Vincent was born on December 26, 2002. Already having three children, we approached the day with the ease that experience brings. What we didn’t anticipate would be any parents’ nightmare. At 10:10 am, on this snowy day, our lives would change forever. He arrived with a cry that was so soft it was barely audible. The surgeons and nurses were busy around the OR, but the silence was deafening. By 10:20, my husband, Frank, sat down next to me, shut the video camera off and stared in silence. He was unable to let his eyes meet mine, yet I knew by his face that something was horribly wrong. I remember asking him “What is the matter?”, but he wouldn’t answer me. He couldn’t. He didn’t have to.
Like all other newborns, Vincent entered this world blue. Normally, as the babies’ lungs fill with oxygen and then circulate it through the body, they turn a healthy pink. Vincent never did. Despite all of their efforts, he became known in the NICU as the “little blueberry”. After waiting what seemed a lifetime we were brought to the NICU to see our son being wheeled off in an incubator hooked up to oxygen. He was immediately transported to Columbia Presbyterian Babies Hospital in NYC. Only 5 ½ hours old Vincent had his first surgery with his dad by his side.
Still at the hospital in NJ, I received a phone call while still in recovery for my C-section. The voice on the other end of the phone explained that my son had a heart defect known as “d-transposition of the greater vessels”. Due to this defect, the two major arteries leaving the heart are connected to the wrong ventricles (lower chambers). The result is that the blood receiving oxygen from the lungs is pumped right back into the lungs. Therefore, the blood that lacks oxygen, which is necessary to nourish the body and organs, is pumped throughout the rest of the body. The voice told me that if they did not help him he would be what is known as a “blue baby” and he would eventually die. Fighting back tears, I gave this voice on the phone “verbal consent” to perform the first surgical procedure needed to save my baby, one that I had never held, or touched and had only seen a glimpse of.
After several hours of battling with the hospital/insurance protocol in NJ, I assured them that if they did not arrange to transfer me to the same hospital as my baby, I would leave against medical advice. Countless hours and an ambulance ride later, I arrived at Columbia Presbyterian Babies Hospital to meet my husband. At approximately midnight, I finally got to see the baby that I gave birth to 14 hours earlier.
At 4 days of age, Vincent had open heart surgery. The procedure that he needed to have is known as the “Arterial Switch” operation. We were extremely fortunate to be assigned to the world renowned surgeon, Dr. Jan M. Quaegebeur, MD who created and perfected this very procedure. The surgery went off without a hitch. When they were done, we were brought to our tiny baby of 6 lbs that we didn’t even recognize. He was totally bloated from the surgery. He was intubated and using a respirator. He had a midline incision down the center of his chest where they had cracked his sternum to perform the procedure and a chest tube to make sure that the incision drained. IV lines came from both arms and both legs. Wires and tubes were everywhere and although he was heavily sedated, he still looked as if he wanted to be comforted by a set of loving arms. As I fought with the instinct to pick him up, I was told that if I touched him it would only stimulate him and make him aware of his surroundings. I sat endless amounts of time holding his tiny foot. It was one of the only parts of him that wasn’t hooked up to something. My husband and I spent that New Year’s Eve in the NICU hoping to see our son open his eyes and thanking God for modern medicine.
About a week later, Vincent was transferred back to the local hospital where he was born. He needed to stay in the NICU there until they were sure that he was eating and healing properly enough to come home. Finally, however, I was allowed to pick him up and hold him. After a few days, they sent us home and advised us to “get on with life and forget that this nightmare had ever happened.”
Several check-ups with pediatricians and cardiologists later, we were assured that Vincent’s heart was better than perfect. There wasn’t even any sign of the murmur that is usually common after heart surgery. Slowly and apprehensively we did get on with life. Remember that we also had three other children at home ages 9, 7, and 4. Much of that year we spent away from the public and only allowed a few family members at a time to visit. Anyone with sick children or a cold themselves knew not to come.
As Christmas came again, we celebrated Vincent’s 1st birthday. There was no party. We didn’t want to expose him to a lot of people. Our family was thankful yet happy to have the year come to an end. With the exception of some physical and emotional scars, we were our perfect little family again and looking forward to starting over with the New Year.
Having put the year behind us we decided to go away as a family. We wanted to try to make up for some of the lost family time that had affected us all. We set off for Florida to spend Valentine’s Day with Nonna. We returned home on a Friday relaxed and refreshed. Monday, we took Vincent for his 1 year post operative cardiology check up. This doctor visit was not required; however, my husband and I decided to take him in just to be safe.
Dr. Zvi Marans, Pediatric Cardiologist, greeted us with smiles and compliments at how wonderful Vincent looked. He did the routine check-up commenting on how great his heart sounded. Then, as always, came the EKG. Dr. Marans became very quiet. After a few moments, he told us that the EKG did not look normal. The reading was “pulling to the right”, as he put it and so he suggested an Echocardiogram to be thorough. Sure enough, the echo showed that the right side of Vincent’s heart was very thick.
Dr. Marans showed us how his information was indicating that the right side of Vincent’s heart was working noticeably harder than the left. As the doctor started to explain what he suspected could be wrong, I felt our perfect little world slip away all over again.
Dr. Marans believed that Vincent could be suffering from a very rare but fatal condition known as Pulmonary Hypertension. Pulmonary Hypertension is a blood vessel disease of the lungs in which the pressure in the pulmonary artery rises above normal levels to become life threatening. In order to diagnose the condition for sure, we had to take him in for a heart catheterization. This procedure is done by inserting a catheter in the main artery found in the groin. The catheter is then fed up through the body until it enters the chambers of the heart. While there the catheter is able to measure the amount of pressure in the arterial passages of the heart. The soonest appointment we were able to get was 12 days later. Totally in denial, after fearing the worst and hoping for the best we returned again to Columbia Presbyterian Babies Hospital, a place we hoped that we would never have to return to again. On March 1, 2004 our worst fears came to fruition.
It was confirmed that our son indeed was suffering from severe Pulmonary Hypertension. The doctor then explained how normal arterial pressures should range from 25-35 mm Hg depending upon whether or not you are resting or exercising. At rest, Vincent’s pressures were 135 mm Hg. They were so high that his heart is in a constant state of heart failure. Most people think that heart failure means that your heart has stopped. In fact, what it means is that the heart is always working much harder and faster than it should be, and because of this, it doesn’t work efficiently. Eventually all of the stress will tire the heart until it stops. Then came the worst part of the entire nightmare. They told us that “there is no cure for this disease”. We were given two options: we needed to try to lower the pressures in Vincent’s heart or he have a double lung transplant. Besides being too young at the time, there is a host of other criteria needed to be met in order to be a transplant recipient. Although transplantation could get rid of the pulmonary hypertension, we would only be trading in one set of problems for another.
To look at Vincent at the time of his diagnosis one would never imagine him to be sick at all. He is at a perfectly normal height and weight. He eats like a trooper, sleeps like a log and always wears a smile as he tries his best to keep up with his older sister and two older brothers. Although beneath his clothing his tiny body is covered with scars from the chest down, he doesn’t seem to have a worry in the world. Having to accept this illness that we couldn’t “see” made the situation even harder to deal with. You find yourself questioning “why my child?” and “what did I do wrong along the line?” Friends and family gathered together to help in any way possible at the same time knowing that there really wasn’t anything they could do to stop the pain that my husband and I were experiencing.
Vincent was assigned to the Pulmonary Hypertension Team, at the Children’s Hospital of New York (CHONY). He was put under the direct care of Dr. Erika Berman- Rosenzweig, MD and the entire pulmonary team of CHONY. Vincent was put on several medications right away. Digoxin for his heart, Lasix , a diuretic and a vasodilator called Flolan. Flolan is a continuous intravenous infusion which is pumped into his body 24 hours a day 7 days a week. It is a vasodilator, which basically means that it opens the arteries and allows the blood to rush through. In order to leave the hospital Vincent needed to have a Broviac Catheter placed in his chest so that the Flolan could be given to him constantly. This catheter comes out of his body in the chest area. It is then, tunneled under the skin and into one of his major arteries where it follows the path into Vincent’s heart. Inside, the drip delivers the medication in an effort to keep his tiny blood vessels dilated as much as possible. By doing so, we hope to lower his arterial pressures and relieve as much stress as we can from his already overworked heart.
Flolan is only active in your blood for 6 minutes. In order to keep the medication flowing, a 100ml cassette filled with Flolan is attached to a pump. The pump advances at a specified rate according to his bodies needs.
At age one, at the time of diagnosis; Vincent needed to carry this pump on his back. The pump in addition to the 100ml cassette goes from the back of his neck to the small of his back. He needs to wear a chest harness with a backpack in order to be mobile independently. The Flolan (medication) also needs to be kept cold so he must lug around icepacks in the backpack as well. Remember, at the time he was only 1, had only been walking a short time and weighed only 20lbs. While trying to deal with this misfortune life had dealt us, I had to attend classes to learn how make Vincent’s daily doses of medication. Once activated by a liquid buffer, the powdered Flolan that we receive is good for only 24 hours if it is kept cold. Therefore, each month we place an order for and receive everything from soup to nuts needed to take care of him at home. I had to learn how to sanitize and sterilize a work area and all of the items needed to make what we refer to as “the mix”. So sensitive is this drug that if it is not kept at the right temperature it is only effective for 8 hours. It is heat sensitive and light sensitive and must be mixed in a specific way. Vincent’s dose must be increased every so many days until such time that his body can longer tolerate the dose. Once this occurs, then we know what dosage he needs to receive. He may never reach a limit. With every increase there are side effects. They range from fatique, headaches, jaw pain, foot pain, muscle pain in calves, nausea, vomiting, diarrhea, flushing, a blotchy facial and body rash, joint discomfort, abdominal pain and photosensitivity to name a few. Dr. Berman-Rosensweig and her assistants, as well as the pulmonary nursing staff, gave us a crash course in this disease and the treatment surrounding it. A Theracom, Inc. representative, Mrs. Jan L.Boland,RN, was flown in to teach me everything there was to know about the medication, ordering it, and getting insurance to pay for it. Jan taught me how to make the mix, run the pump, clean his site, and keep my spirits up. Looking back now, everyone involved was truly wonderful. They taught us a lot of information in a very short period of time and tried to be mindful of the shock we were in having gotten this diagnosis.
We learned that we must always have a backup mix and pump on hand. In the case of an emergency, the medication already in Vincent’s body is only good for 6 minutes. We must have all of his medical supplies and a backup ready at any given moment. If this isn’t bad enough the catheter in Vincent’s body is highly susceptible to infection. Keeping the exit site and the indwelling catheter “infection free” is of constant concern. Try explaining to a one year old that he must lie still for 20 minutes and allow mommy to pull off the adhesive holding his catheter in place. Imagine pulling a bandage and tape off of the sensitive skin of your chest. Now picture explaining to a one year old that you are going to do this and he can’t move! We were taught a specific procedure for taking care of the site. Gloves and masks must be worn to ward off germs after scrubbing with surgical scrub for a full three minutes. For awhile, it took me and my mother or Frank to do this because we needed to keep him calm and at the same time make sure that he didn’t touch the area at all. The biggest problem with the catheter, besides infection, is if it snaps or breaks. We have 6 minutes of his medication working. After 6 minutes anything can happen. There have been people who have gone into immediate cardiac arrest. Vincent can turn blue, pass out, and go into shock just to name a few. When this occurs they need to put a peripheral IV into Vincent so that we can get his meds into his body. The need for access being so important and the Flolan being so strong means that two different IV sites are needed at all times. Trying to get an IV into the vein of a one year old is close to impossible. Imagine trying to get two lines in. Several times, Vincent has completely turned blue and passed out in the process. This type of stress on a pulmonary system that is already at risk can be fatal.
By April of 2005, we had come full circle. In the year since he had been diagnosed, (not counting the original three weeks we spent at Columbia) Vincent has had two minor line infections requiring oral and topical antibiotics, has had two major line infections which required hospitalization and intravenous antibiotics. He had one catheter fall out completely. Two catheters cracked and needed to be repaired and two snapped all together. Each time a catheter needed to be replaced, a surgery and several days in the hospital were needed. All of these circumstances meant trips to the emergency room in NYC. Several of these trips by way of ambulance because of the “6 minute rule”.
Any sign of a cold, or temperature for any reason must be treated vigorously. The reason being is that a fever could be a sign of infection. If the infection reaches the blood stream it can become sepsis and he can possibly die. A common cold for you or me, for Vincent, could easily turn to bronchitis or pneumonia. His lungs are so compromised that his body can not handle such a sickness. Some PH patients who had otherwise been doing fine ended up getting pneumonia and dying. Because of this alone, we avoid large groups of people like the plague. My husband and I take turns staying home with Vincent while the rest of our family members attend gatherings. We have missed countless birthday parties and other events for fear of Vincent being exposed to something especially from little children. We have even had to tell people that he must come to their wedding with us or we cannot attend. We cannot leave him with a babysitter. Even if we wanted to try, everyone is afraid that something might happen while we are not there. Every year our entire family must have the Flu shot. Vincent also receives two different pneumonia shots as precaution. He has also gone through a sleep study (also in the hospital) which determined that he had severely obstructed oxygen intake while he sleeps. This led to yet another surgery to remove both his tonsils and adenoids. We never know what tomorrow will bring.
Our other three children have had to grow accustomed to not knowing if Vincent and I will even be home when they return from school. They each know all the components needed to make “the mix” and how to program his computerized pump as well as trouble shoot its system in case the alarm sounds. We all keep constant watch on his ice packs and his dosing schedule for all of his meds.
Our family has adjusted to “making the mix” and working the pump on a daily basis. We have an entire room designated to make his meds and to house the large supply of medical supplies which must be on hand at all times. They know that they must wash their hands constantly and to stay away from Vincent if they have even a hint of a cold coming on. There have been times when they are sick that I make them wear a mask if they are not in their own rooms. When we do leave the house as a family we must bring his backup and extra icepacks in a small cooler and a bag full of supplies at all times. Although it can be bothersome, we have accepted that this must be done. In our home, there is always a suitcase for Vincent and I packed and ready to go. We have all been programmed to expect “total chaos” at any moment.
What you don’t grow accustomed to is the not knowing. Every holiday that passes I wonder if he will make it to the next. Will we plan his future or his funeral? This feeling never leaves me. Vincent is one of the lucky ones. To date, he is
A symptomatic. Most people with PH can not walk a flight of stairs without losing their breath. A lot of PHer’s need constant oxygen, some use wheelchairs and are in a constant state of fatigue. They become dizzy, gasp for air and have syncope episodes. (passing out) Will Vincent stay without symptoms? This is just another unanswered question that our family lives with everyday.
I went to a support group session and was advised that I need to come to terms with the fact that “everyday when you wake up, you need to realize that today might be Vincent’s last day alive.” This is not something that I can become accustomed to nor do I want to. I spend every minute of every day trying to keep him as strong and healthy as possible. I have had to ask my other children to understand time and time again that I need to spend more time with him and less time with them than they have always been accustomed to. Even at their young ages they have each expressed to me that they fear losing Vincent all together. To them, he is simply their baby brother. They don’t care if he wears a backpack or has a rash on his face from time to time. They have welcomed him into our family with open arms. None of us can even bear the thought of him not being a part of it.
Through his siblings, Vincent has become a rough and tough little boy with the smile of an imp and the heart of an angel. To me, he is my little hero. I have yet to meet anyone half as courageous, accepting and forgiving as he is and he is only three. No matter what each day brings he always faces it with a huge smile. He truly is amazing.
On January 5, 2006 he had another heart catherization to see if all we have gone through so far was working or not. There was some improvement but not enough to make a real difference. We then added another medication to his daily regiment and continued to deal with his disease everyday. If all of this wasn’t enough for this little boy to have to go through, all the medications he is on started to rot Vincent’s teeth. At the age of three, he needed to have oral surgery to remove most of his teeth because the chance of infection was too high a risk for us to take.
Luckily, 2006 was a year that meant no troubles with his PH and Vincent prospered every day. He is growing, eating and sleeping better than we expected and rough houses with his older brothers and sister a little more than we would like. What can you do, “boys will be boys” and we’d like to let Vincent have as normal a life as possible. Please keep him in your prayers and help us hope for the best.
Vincent can not win his battle alone. What needs to be accomplished is that people need to be aware that a disease like this even exists. A cure needs to be found, not only for Vincent, but for the thousands of PH patients in the world today. Pulmonary Hypertension must become a less hidden illness. With every new person we contact and educate our chance to make a change increases. It has been said, “For every 100 people who hear our story there are four or five in unique positions to advance our common cause.” The need for public awareness is vital. The vast majority of our medical professionals do not even know what PH is. That is frightening.
However, an even bigger contribution can be made after you have taken the time to read this. Make copies of this story and pass it on to as many people as you can. Just tell us where to send our story so the word can continue to be spread.
Pulmonary Hypertension is unknown to most people. There is a website www.phassociation.org which is very informative and open to all. PH needs to be made a household word. Even friends and extended family members of PH patients don’t really know what it is like to live with PH. We have given you just a small glimpse into our world. Please help us make as many people aware of Pulmonary Hypertension as possible. With your help perhaps this uphill battle can become a little easier.
THE ACCURSO FAMILY
Frank, Nadine, Anthony, Nicholas, Marissa and Vincent